Ocular motor dysfunction in Parry-Romberg syndrome: four cases.

The authors describe four patients with Parry-Romberg syndrome (PRS) who had abnormal eye movements, ptosis, and facial hemiatrophy. There were delays in diagnosis in all cases, often requiring multiple imaging studies, prior to diagnosis of PRS. These cases demonstrate the variable ophthalmic manifestations of PRS, which can lead to difficulty in diagnosis. Case 1 was a 23-year-old woman with lateral rectus paresis who presented with horizontal diplopia and developed ptosis and facial hemiatrophy. Case 2 was a 28-year-old woman with medial rectus weakness adjusted to diplopia by head turn and gaze preference. Case 3 was a 68-year-old woman who presented with dry eye symptoms and was noted to prefer right gaze and have left hemifacial atrophy. Case 4 was a 68-year-old woman who presented with ptosis, enophthalmos, and restriction of up gaze. The ocular motor defect in all of these cases is best explained by muscle fibrosis rather than nerve paresis.

Transient visual loss in a 60-year-old man.

A 60-year-old man after 5 years of recurrent episodic amaurosis fugax, always resolving, experienced an incident of visual loss that was permanent. Computed tomography and magnetic resonance imaging were normal. Transesophageal echocardiogram and carotid duplex scan ruled out embolic sources from the heart and neck vessels. Blood tests for hypercoagulability found elevated APTT not correcting with 50:50 dilution with control serum, owing to the presence of a lupus anticoagulant. The patient was treated with warfarin, baby aspirin, and a calcium channel blocker and has not had a recurrence of permanent visual loss in 17 years.

Concurrent sino-orbital aspergillosis and cerebral nocardiosis.

A 79-year-old man with myelodysplastic syndrome developed a right optic neuropathy with optic disc edema and intractable periocular pain, one month after undergoing removal of a gangrenous gallbladder. Although results of a temporal artery biopsy were negative, he was treated with prednisone for presumed temporal arteritis. Attempts at tapering the prednisone dose led to recurrence of periocular pain. On neuro-ophthalmologic evaluation six months after the prednisone treatment was begun, he had developed right fourth and sixth cranial nerve palsies, and magnetic resonance imaging demonstrated a right orbital apex mass. Trans-sphenoidal biopsy revealed Aspergillus fumigatus. During treatment of aspergillosis, the patient developed a left hemiparesis. Magnetic resonance imaging disclosed multiple ring-enhancing cerebral masses. Biopsy revealed Nocardia asteroides. The patient was successfully treated for both infections with recovery of neurologic function except for the right optic neuropathy. Although immunocompromised patients are known to be subject to multiple infections, this may be the first reported case of concurrent sino-orbital aspergillosis and cerebral nocardiosis.

The pathogenesis and treatment of optic disc swelling in neurosarcoidosis: a unique therapeutic response to infliximab.

OBJECTIVE: To review the pathogenesis and treatment of optic disc swelling in neurosarcoidosis, including a novel therapeutic response to infliximab. DESIGN AND SETTING: Case reports from an inpatient neurology service. PATIENTS: A 35-year-old woman presented with headache, chronic visual loss, papilledema, and optic atrophy, characteristic of chronic intracranial hypertension. Magnetic resonance imaging showed bifrontal cerebral edema with en plaque frontal pachymeningeal enhancement. Her visual loss progressed despite conventional therapies. The use of the tumor necrosis factor alpha antagonist infliximab maintained functional vision in her right eye. A 57-year-old woman presented with bilateral, subacute, painful visual loss and unilateral papillitis consistent with optic neuritis. Her visual loss responded rapidly to intravenous corticosteroids. The funduscopic examination findings in both patients prompted further clinical investigation, culminating in the diagnosis of neurosarcoidosis. CONCLUSION: Understanding the multiple etiologic mechanisms that produce optic disc swelling in sarcoidosis can help neurologists tailor treatment for patients with neurosarcoidosis who present with this symptom.

Anterior ischemic optic neuropathy in children: case reports and review of the literature.

Anterior ischemic optic neuropathy, infarction of the optic nerve head owing to inadequate perfusion through the posterior ciliary arteries, is a common cause of visual loss in adults but is rarely reported in children, in part because the diagnosis is overlooked. We report two cases of young children undergoing chronic peritoneal dialysis, who suffered bilateral visual loss from anterior ischemic optic neuropathy. Predisposing local anatomic and multiple systemic factors included a small optic nerve head with little cupping, possible intraocular hypertension, and systemic hypotension, hypovolemia, and anemia. The literature on anterior ischemic optic neuropathy is reviewed.